Generation of an Inbred Miniature Pig Model of Retinitis Pigmentosa

Ross, Jason W.; Castro, Juan P. Fernandez de; Zhao, Jianguo; Samuel, Melissa; Walters, Eric M.; Rios, Cecilia; Bray‐Ward, Patricia; Jones, Bryan W.; Marc, Robert E.; Wang, Wei; Zhou, Liang; Noel, Jennifer; McCall, Maureen A.; DeMarco, Paul J.; Prather, Randall S.; Kaplan, Henry J.

doi:10.1167/iovs.11-8784

Cited by 133 publications

(123 citation statements)

References 18 publications

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“…Genetic ablation of retinoid storage by knocking out lecithin retinal acyltransferase in P23H/ϩ knock-in mice significantly accelerates the progression of retinal degeneration due to the P23H mutation (24). Despite the protective effect of endogenous chromophore, all previous animal models, including P23H TG Drosophila (14), Xenopus (10,58), rats (12), mice (11, 59 -61), pigs (13), and knock-in mice (24,25), undergo various degrees of retinal degeneration that are also reported in adRP patients carrying this mutation (24,62). This suggests that the presence of the endogenous chromophore does not suffice to prevent P23H-induced adRP.…”

Section: Discussionmentioning

confidence: 99%

Inherent Instability of the Retinitis Pigmentosa P23H Mutant Opsin

Chen

Jastrzębska

Cao

et al. 2014

Journal of Biological Chemistry

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Background:The P23H opsin mutant causes the blinding human disease, retinitis pigmentosa. Results: Molecular properties of bovine P23H mutant opsin were characterized in both in vitro and a transgenic C. elegans model. Conclusion: Thermally unstable P23H isorhodopsin containing correct disulfide bond can be slowly regenerated in transgenic C. elegans. Significance: This study produced novel information about the disease-causing P23H mutant opsin.

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Section: Discussionmentioning

confidence: 99%

Inherent Instability of the Retinitis Pigmentosa P23H Mutant Opsin

Chen

Jastrzębska

Cao

et al. 2014

Journal of Biological Chemistry

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“…This is presumably due to the ocular inflammation that generally causes damage to the transport pump and thereby prolonging the half-life of drugs that are eliminated by the posterior (retinal) route. After intravitreal injection in rabbits, the vitreal half-life of ketorolac in normal eyes was 2.28 h (Wang et al 2012) while the half-life was 4.27 h (Baranano et al 2009) in eyes with ocular inflammation. Similar results were reported by other authors where the vitreal half-life of ceftriaxone (Jay et al 1984) and cefazolin (Ficker et al 1990) was longer in the aphakic eye when compared to phakic eyes.…”

Section: Influence Of Disease State On Ocular Pharmacokineticsmentioning

confidence: 99%

Corticosteroids and Anti-Complement Therapy in Retinal Diseases

Narayanan

Kuppermann

2016

Handbook of Experimental Pharmacology

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“…European pig breeds, such as the Yorkshire, Landrace, and Duroc, are noted for their lean meat content and fast growth rate, but the litter size is poor, meat quality is not as good as local pig breeds [2]. Pig farming has application in animal husbandry, at the same time, the pig has been used as a model in many biological [3], agricultural, and biomedical studies [4][5][6].…”

Section: Introductionmentioning

confidence: 99%

Molecular cloning, sequence characteristics, and tissue expression analysis of the SLC35D3 gene in lean, obese and mini-type pigs

Liu

Zhou

et al. 2016

Preprint

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Abstract：Nowadays, with the development of people's life, obesity become one of the largest health problems today. Solute carrier family 35, member D3 (SLC35D3) protein has been reported to be involved in adipose deposition and metabolic control in mice. Because organ size in pig is comparable to that of the human, the pig is an ideal model that has been used to study diabetes mellitus, atherosclerosis, obesity, and other diseases. To better understand the structure and function of the SLC35D3 gene, the Meishan pig SLC35D3 gene was cloned and characterized, and its expression in different tissues was determined. The SLC35D3 cDNA consisted of a 1272 bp coding sequence that encoded a protein of 243 amino acids with a molecular mass of 44653.9 Da, and 966 bp 3′ untranslated regions. It is a pity that we have not got 5′ untranslated regions. Phylogenetic tree analysis revealed that porcine SLC35D3 had a closer genetic relationship and a shorter evolutionary distance with Vicugna; however, its evolutionary distance with that of the gorilla was longer than that of Vicugna. In addition, we quantified the SLC35D3 mRNA level by real-time polymerase chain reaction and detected expression in the liver, kidney, lung, heart, brain, LM, and spleen, as well as in the leaf lard, SAT, and PAT. In addition, we also tested the expression level of Meishan, Bama and Yorkshire in adipose tissue. The SLC35D3 mRNA level was highest in the leaf lard. These results serve as a foundation for further study on the porcine SLC35D3 gene.PeerJ Preprints | https://doi.org/10.7287/peerj.preprints.2661v1 | CC BY 4.0 Open Access |

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Generation of an Inbred Miniature Pig Model of Retinitis Pigmentosa

Cited by 133 publications

References 18 publications

Inherent Instability of the Retinitis Pigmentosa P23H Mutant Opsin

Inherent Instability of the Retinitis Pigmentosa P23H Mutant Opsin

Corticosteroids and Anti-Complement Therapy in Retinal Diseases

Molecular cloning, sequence characteristics, and tissue expression analysis of the SLC35D3 gene in lean, obese and mini-type pigs

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